Multiple system atrophy with hippocampal pathology.
Multiple system atrophy with hippocampal pathology.
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DOI：
10.1111/bpa.13067
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发表时间：
2022-05
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Ando等人[1]在最近对来自日本多家医院的146例经尸检证实的多系统萎缩(MSA)患者(男性82例，女性，发病年龄58.5±9.3/33-83岁)中发现12例(8.2%-7 MSA-P和5 MSA-C)患者患有严重的海马区病理，其原因是α-突触核免疫反应神经元胞浆内包涵体(NCI)显著受累于严重的神经元丢失和星形胶质细胞，主要位于海马区颗粒细胞、CA1/小脑、海马旁回和杏仁核。NCIS呈环状或神经原纤维缠结(NFT)样构型。此外，12例患者中有3例表现为不典型的Pick体样NCI，内侧颞叶严重萎缩，NCI受累严重。1例(8.3%)脑干可见路易小体，表现为神经元性Braak NFT分期I~III期(平均1.6±0.8)，略高于经典MSA。海马区MSA变异型患者的发病年龄(平均56.6±9.3年)比典型MSA患者(60.4±9.3年)更早，病程(平均13.2±5.9年比6.9±3.6年)更长，认知障碍的患病率更高，额颞叶皮质NCIS密度更高。这种海马亚型被认为是MSA的一种罕见的病理变异，而不是晚期疾病的结果。在奥地利维也纳临床神经生物学研究所的48例尸检确诊的MSA病例中(33例MSA-P和15例MSAC)，平均发病年龄为55.5±6.5岁，平均病程为7.5年，其中2例女性，均为MSA-P，均有严重的海马区病理改变，分别在61岁和73岁起病。他们占总队列的4.3%。从确诊到死亡的时间分别为4年和7年。两例患者的首发症状均为僵硬、运动迟缓和步态障碍，无震颤或本质自主神经症状(立位性低血压、排尿困难等)，而年长的患者出现轻微的小脑症状。临床诊断为帕金森病R-A型和MSA-P，上次就诊时Hoehn&Yahr分期为4~5级。老年患者出现精神病症状、视觉幻觉、抑郁和中度认知障碍，后来出现喉鸣，最后接受了聚乙二醇仪。年轻的孩子除了帕金森症状外，还出现了轻度肌肉萎缩和中度认知障碍(MMSE 25/30)。两个病例的神经病理均显示额叶和海马区萎缩，纹状体黑质变性III级[2]，老年患者OPCA I级。除纹状体、脑干和大脑皮层有多个α阳性的胶质细胞质包涵体(GCI)外，NCI受累严重，主要表现为神经元丢失和星形胶质细胞增生，主要分布在海马区CA/1区、颗粒细胞层、前丘脑和前丘脑，以及海马旁回。与Ando等人[1]报道的病例相反，在脑干中没有观察到Pick小体或NFT样NCIS和Luy小体，而在MSA队列中23%的人看到了Louy病理。两例均表现为中度的海马tau病变，类似于Braak NFT III期，Thal淀粉样蛋白0-3期(平均0.8±0.1)。没有观察到星形胶质细胞、大脑淀粉样血管病变、TdP-43共同病理，也没有边缘型和FTLD型α-突触核蛋白病理[3]。两名患者的精神病性症状和认知障碍被认为与…严重累及海马区有关
Ando et al.[1], in a recent study of 146 autopsy-proven cases of multiple system atrophy (MSA) from various Japanese hospitals (82 males, 64 females, aged at disease onset 58.5±9.3/33–83/years) identified 12 patients (8.2%-7 MSA-P and 5 MSA-C) with severe hippocampal pathology due to prominent involvement by α-synucleinimmunoreactive neuronal cytoplasmic inclusions (NCIs) associated with severe neuronal loss and astrogliosis predominantly in the hippocampal granule cells, the CA1/subiculum, parahippocampal gyrus and amygdala. The NCIs showed ring-shaped or neurofibrillary tangle (NFT)-like configurations. In addition, 3 of the 12 patients showed atypical Pick body–like NCIs and severe atrophy of the medial temporal lobes with heavy NCI involvement. In addition, Lewy bodies in the brainstem were seen in one (8.3%) of the hippocampal MSA cases, which showed neuritic Braak NFT stages I-III (mean 1.6±0.8), which was slightly higher than in the classical MSA cases. The patients with the hippocampal MSA variant were younger at disease onset than the classical MSA cases (mean 56.6±9.3 vs. 60.4±9.3 years), had a significantly longer disease duration (mean 13.2±5.9 vs. 6.9±3.6 years) and higher prevalence of cognitive impairment, associated with denser NCIs in the fronto-temporal cortex. This hippocampal subtype was considered a rare pathological variant of MSA and not a result of an advanced disease phase. Among 48 autopsy-confirmed cases of MSA from the files of the Institute of Clinical Neurobiology, Vienna, Austria (33 MSA-P and 15 MSAC) with mean age at disease onset of 55.5±6.5 years and a mean duration of 7.5 years, two females, both MSA-P with severe hippocampal pathology, showed disease onset at 61 and 73 years, respectively. They accounted for 4.3% of the total cohort. The time from diagnosis to death was 4 and 7 years, respectively. In both patients, the initial symptoms were rigidity, bradykinesia, and gait disorders, without tremor or essential autonomic symptoms (orthostatic hypotension, urinary difficulties, etc.), while the elder one developed mild cerebellar symptoms. The clinical diagnosis was Parkinson disease R-A type and MSA-P, respectively, with Hoehn & Yahr stage 4 to 5 at last visit. The elder patient developed psychotic symptoms, visual hallucinations, depression, and moderate cognitive impairment, later laryngeal stridor, and finally received a PEG probe. The younger one, in addition to parkinsonian symptoms, developed mild muscular atrophy and moderate cognitive impairment (MMSE 25/30). Neuropathology in both cases revealed frontal and hippocampal atrophy, striatonigral degeneration grade III [2], with OPCA grade I in the elder patient. In addition to multiple α-synuclein-positive glial cytoplasmic inclusions (GCIs) in the striatum, brainstem and less in cerebral cortices, there was severe involvement by NCIs with neuronal loss and astrogliosis predominantly in hippocampal subarea CA/1, granular cell layer, pre-and prosubiculum, and parahippococampal gyrus. In contrast to the cases reported by Ando et al.[1], no Pick bodyor NFT-like NCIs and no Lewy bodies in the brainstem were observed, while Lewy pathology was seen in 23% of the total MSA cohort. Both cases showed moderate hippocampal tau pathology akin to Braak NFT stages III, with Thal amyloid phases 0–3 (mean 0.8±0.1). No taupositive astroglia, cerebral amyloid angiopathy (CAA), TDP-43 co-pathologies, and no limbic and FTLD-type α-synuclein pathology [3] were observed. The psychotic symptoms and cognitive impairment in both patients were suggested to be correlated to the severe hippocampal involvement …
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影响因子：
5
作者：
Coughlin, David G.;Dryden, Ian;Goodwill, Vanessa S.;Pizzo, Donald P.;Wright, Brenton;Lessig, Stephanie;Galasko, Douglas;MacKenzie, Ian R.;Hiniker, Annie
通讯作者：
Hiniker, Annie
影响因子：
3.3
作者：
Jellinger, Kurt A.
通讯作者：
Jellinger, Kurt A.
影响因子：
8.6
作者：
Jellinger, KA;Seppi, K;Wenning, GK
通讯作者：
Wenning, GK
DOI：
10.1111/bpa.13002
发表时间：
2022-01
期刊：
Brain pathology (Zurich, Switzerland)
影响因子：
--
作者：
Ando T;Riku Y;Akagi A;Miyahara H;Hirano M;Ikeda T;Yabata H;Koizumi R;Oba C;Morozumi S;Yasui K;Goto A;Katayama T;Sakakibara S;Aiba I;Sakai M;Konagaya M;Mori K;Ito Y;Yuasa H;Nomura M;Porto KJL;Mitsui J;Tsuji S;Mimuro M;Hashizume Y;Katsuno M;Iwasaki Y;Yoshida M
通讯作者：
Yoshida M